liver part 1 cont.

Question 1

clinical features of portal vein Obstruction and Thrombosis

Answer 1

• Abdominal pain
• Ascites
• Oesophageal Varices
• Acute impairment of visceral blood flow –>
  Congestion and Bowel Infarction

Question 2

cause of Portal Vein Obstruction and Thrombosis

Answer 2

Impaired Blood Flow
Into the Liver

Question 3

casue of Impaired blood flow Troughthe liver

Answer 3

– Cirrhosis (most common intra-hepatic cause)
– Sickle Cell disease: Obstruction of the sinusoids
by sickled red cells –> Pan-lobular parenchymal
necrosis
– Disseminated Intravascular Coagulation (DIC) –>
Occlusion of sinusoids

Question 4

casue of Passive Congestion &
Centri-Lobular Necrosis

Answer 4

Systemic circulatory disorders:
* Right-sided cardiac Decompensation –> Passive
Congestion of the Liver
* Long-standing cases –> Centri-Lobular necrosis
and peri-venular fibrosis in the necrotic areas

Question 5

Laboratory findings:
– Mild elevation of serum transaminase levels
– Hyper-bilirubinaemia and elevated alkaline
phosphatase (some cases)

Findings of what type of Condition?

Answer 5

Passive Congestion &
Centri-Lobular Necrosis

Question 6

Macroscopic features:
– Slightly enlarged
– Tense
– Cyanotic Liver

Microscopic findings:
– Congestion of Centri-Lobular sinusoids
– Atrophic Centri-Lobular Hepatocytes –> Markedly attenuated Liver cell cords
– In continuous chronic severe Congestive Heart Failure
–> “Cardiac Sclerosis” or “Cardiac Cirrhosis”:
Mainly, development of centri-lobular fibrosis, with rarely formation of bridging fibrous septa and Cirrhosis

Features of what type of condition?

Answer 6

Passive Congestion & Centri-Lobular Necrosis casued by Right-sided Cardiac Failure

Question 7

Macroscopic picture:
– Variegated mottled appearance, with Centri-Lobular
localised areas of haemorrhage and necrosis,
alternating with pale mid-zonal areas –> “Nutmeg”
liver
-Centri-lobular region is suffused with red blood cells

Answer 7

Centri-Lobular Haemorrhagic
Necrosis (Nutmeg Liver) –> casued by Left-sided hypo-perfusion + Right-sided retrograde congestion

Question 8

cause of Hepatic vein Thrombosis aka Budd-Chiari Syndrome

Answer 8

Polycythaemia Vera, Pregnancy, use of Oral Contraceptives, Paroxysmal Nocturnal
Haemoglobinuria, Hepatocellular Carcinoma; 10% of
cases idiopathic

Question 9

Clinical features of Hepatic Vein Thrombosis akaBudd-Chiari Syndrome

Answer 9

– Hepatomegaly
– Ascites
– Abdominal pain

Question 10

Macroscopic features:
– Swollen and red-purple liver
– Tense organ capsule

Microscopic findings:
– Severe Centri-Lobular congestion and necrosis
– Centri-Lobular fibrosis (when Thrombosis develops
more slowly)
– Completely or incompletely occlusive fresh Thrombi in the lumen of Major Veins
– Organised adherent Thrombi (chronic cases)

Features of what type of Condition?

Answer 10

Hepatic Vein Thrombosis

Question 11

Treatment of Hepatic vein Thrombosis

Answer 11

• Surgical creation of porto-systemic venous shunt
  – Angiography for direct dilation of Vena Cava
  obstruction

Question 12

patho of Hepatic Vein Thrombosis

Answer 12

– Majority of the conditions –> Thrombotic
tendencies
– Hepatocellular Carcinoma –> Hyper-coagulability
state (sluggish blood flow)
– Massive intrahepatic Abscess or Parasitic Cyst –>
Mechanical obstruction to blood outflow
– Thrombus or Tumour –> Obstruction of the
inferior Vena Cava, at the level of the Hepatic
Veins

Question 13

cause of Sinusoidal Obstruction Syndrome
aka Veno-Occlusive Disease

Answer 13

Chemotherapeutic agents (e.g. Cyclo-Phosphamide, Actinomycin D, and Mithramycin), and total body
radiation (used in pre- or post-transplantation regimens)

Question 14

patho of Veno-Occlusive Disease (Sinusoidal Obstruction Syndrome)

Answer 14

– Toxic injury to sinusoidal endothelium causes:
–> Sloughing of damaged endothelial cells –> Formation of Thrombi–> Obstruction of sinusoidal flow –> Leak of erythrocytes into the space of Disse
–> Proliferation of stellate cells and fibrosis of the
terminal branches of the Hepatic Vein

Question 15

Clinical presentations of Sinusoidal Obstructive Syndrome

Answer 15

– Onset, in the first 20 to 30 days after bone
marrow transplantation (20% of recipients)
– Resemblance to Budd-Chiari Syndrome, ranging from mild to severe forms

Question 16

casues of Fluminant Hepatic Failure

Answer 16

Viral Hepatitis (HCV > HBV)

Question 17

Macroscopic features:
– Shrinkage of Liver due to massive loss of mass (500-
700gr)
– Limp, red organ
– Wrinkled, large capsule
– Cut surface of necrotic areas: Muddy red mushy appearance with haemorrhage

Microscopic findings:
– Complete destruction of Hepatocytes in neighbouring lobules –> Collapsed reticulin framework and preserved portal tracts
– Little inflammatory reaction, except in cases of survival –> Massive accumulation of inflammatory cells

Are the features of what type of Syndrome

Answer 17

Fulminant Hepatic Failure

Question 18

cause of Focal Nodualr Hyperplasia

Answer 18

Long-term use of Anabolic Hormones or of Contraceptives

Question 19

Epi of focal Nodular Hyperplasias

Answer 19

Young to middle-aged adults

Question 20

Macroscopic features:
* Well-demarcated but poorly encapsulated nodule
* Lighter colouration than the surrounding Liver
* *Central gray-white, depressed stellate scar, with radiations to the periphery

Microscopic findings:
* Large arterial vessels within central scar, showing
fibro-muscular hyperplasia and narrowing of their lumen
* Foci of intense lymphocytic infiltrates within the radiating septa
* Marked bile duct proliferation along septal
margins
* Normal appearing Hepatocytes in the parenchymabetween the septa, but with a thickened plate architecture (typical for regeneration)

Are the features of what type of Condition

Answer 20

Focal Nodular Hyperplasia

Question 21

Macroscopic features:
* Entirely transformed Liver into roughly sphaerical nodules, in the absence of fibrosis

Microscopic findings:
* Plump hepatocytes surrounded by rims of atrophic Hepatocytes (reticulin stain)

Are the features of what type of Condition?

Answer 21

Nodular Regenerative Hyperplasia

Question 22

complications of Nodular Regenerative Hyperplasia

Answer 22

Portal Hypertension

Question 23

loc. of Cavernous Haemangiomas

Answer 23

Directly beneath the capsule

Question 24

Epi of Cavernous Haemangiomas

Answer 24

Most common benign Liver Tumours

Question 25

Macroscopic features:
– Size: <2cm in diameter
– Discrete, red-blue, soft nodules

Microscopic findings:
– Vascular channels in a bed of fibrous connective
tissue

Are the features of which liver tumour?

Answer 25

Cavernous Haemangiomas

Question 26

epi of Hepatic Adenoma

Answer 26

• Benign neoplasm
  – Most common in women that use Oral Contraceptives

Question 27

loc Hepatic Adenoma

Answer 27

Most often, beneath the capsule

Question 28

patho of Hepatic Adenoma

Answer 28

– Mutations in the genes encoding the transcription
factor HNF1α (50% of cases) and β-catenin (15% of
cases)
– Individuals with maturity-onset diabetes of young
(MODY3), with HNF1 mutations –> Multiple Hepatic
Adenoma (Adenomatosis) Syndromes

Question 29

Macroscopic features:
– Size: Up to 30cm in diameter
– Well demarcated lesion
– Pale, yellow-tan, and frequently bile-stained
- Tumour cells resemble normal Hepatocytes or show mild variation in cell and nuclear size
– Abundant intra-cytoplasmic glycogen accumulation –> Large Hepatocytes with a clear cytoplasm
– Commonly, Steatosis
– Absent portal tracts
– Prominent solitary arterial vessels and draining veins

Are the features of what type of liver Tumour?

Answer 29

Hepatic Adenoma

Question 30

Major Aetiologic factors of Hepatocellular Carcinoma (HCC)

Answer 30

– Chronic Viral Infection (HBV, HCV)
– Chronic Alcoholism
– Non-Alcoholic Steato-Hepatitis (NASH)
– Food Contaminants (primarily Aflatoxins)

Question 31

Patho of Hepatocellular Carcinoma

Answer 31

– Development of the tumour from small-cell, high grade dysplastic nodules in cirrhotic Livers
– Dysplastic nodules show mono-clonality and chromosomal aberrations similar to those in HCCs
– Cell of origin of HCCs: Both mature Hepatocytes and progenitor cells (known as ductular cells or oval cells)
– Presence of structural and numeric chromosomal abnormalities in HCCs –> Genomic instability

Question 32

Macroscopic features:
– Patterns of appearance:
1. Solitary mass
2. Multifocal, widely distributed nodules of variable size
3. Diffusely infiltrative Cancer
– Mostly, paler than the surrounding Liver; sometimes,
green hue appearance
– Strong propensity for vascular invasion
– Extensive intrahepatic metastases
– Occasionally, long snake-like masses in Portal Vein (with occlusion of the portal circulation) or Inferior Vena Cava, extending even into the right side of the Heart
Are the features of what type of Liver Neoplasm?

Answer 32

Hepatocellular Carcinoma

Question 33

Histologic findings:
- range from well-differentiated to highly
anaplastic undifferentiated lesions
– Well differentiated and moderately differentiated
tumours: Hepatocyte-like cells; arranged in a
trabecular (similar to Liver cell plates), acinar, or
pseudo-glandular pattern
– Poorly differentiated forms –> Tumour cells can:
* take on a pleomorphic appearance with
numerous anaplastic giant cells
* be small and completely undifferentiated
* even resemble a Spindle Cell Sarcoma

Are the findings of what type of Liver Neoplasm?

Answer 33

Hepatocellular Carcinoma

Question 34

Epi of Fibrolamellar Carcinoma

Answer 34

Young male and female
adults (20-40yrs); F=M

Question 35

Macroscopic features:
- Single large, hard “scirrhous” tumour with fibrous bands

Microscopic findings:
* Well-differentiated polygonal cells, with
eosinophilic cytoplasm and prominent nucleoli
* Arrangement of tumour cells in nests or cords, which are separated by parallel lamellae of dense collagen bundles

Are the features of what type of liver Neoplasm?

Answer 35

Fibrolamellar Carcinoma

Question 36

Clinical Manifestaions of Hepatocellular Carcinoma

Answer 36

– Ill-defined upper abdominal pain
– Malaise
– Fatigue
– Weight loss
– Abdominal mass or abdominal fullness
(sometimes)

Question 37

Physical Examination of Hepatocelluar Carcinoma

Answer 37

Enlarged, palpable Liver,
with sufficient irregularity or nodularity

Question 38

Complications of long-term Fluminant Hepatic Failure

Answer 38

Cirrhosis

Question 39

choice of treatment for Fluminant Hepatic Failure

Answer 39

Liver Transplant